MSUD and illness

During periods of ill health, a person’s energy expenditure automatically increases as the body tries to fight the illness. Appetite might also be affected, causing a reduction in overall energy intake. These two factors combined can result in the breakdown of fat reserves and protein stores from the muscles to compensate and use as energy. This breakdown of protein releases leucine into the blood, causing leucine levels to increase. In MSUD, it is thought to be the inadequate calories as opposed the mechanisms of illness, that cause leucine to rise.

During times of illness, blood concentration of leucine can rise rapidly and an “emergency regimen” (ER) or “emergency feed” is required for individuals with MSUD. For these regimens to work effectively, they must be made up accurately and taken frequently to reduce the accumulation of leucine. Protein intake might also be reduced for a set period to help this process and leucine levels will be measured frequently to determine the rate of protein re-introduction following the illness. The metabolic team will recommend the most appropriate product(s) and give specific guidelines to follow when preparing and administering the emergency feeds. It is important that you store these instructions somewhere safe and that they are followed carefully as the accumulation of leucine can cause you to rapidly become unwell.

Along with the ER, protein substitute should be continued. The amino acids isoleucine and valine may also need to be taken. You will be well prepared by the metabolic team in advance about what to do during times of illness